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累及周围神经的上皮样肉瘤的诊断与治疗。

The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves.

作者信息

Jia Xiaotian, Yang Chaoqun, Chen Lin, Yu Cong, Kondo Tadashi

机构信息

Department of Hand Surgery, Huashan Hospital, Fudan University, Shanghai, 200040, China.

Key Laboratory of Hand Reconstruction, Ministry of Health, Shanghai, 200032, China.

出版信息

Sci Rep. 2024 Dec 28;14(1):31096. doi: 10.1038/s41598-024-82357-z.

DOI:10.1038/s41598-024-82357-z
PMID:39730748
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11680949/
Abstract

Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. The symptoms are similar to those of nerve compression diseases. This situation is extremely rare in clinic and was only reported as several case reports in literature. It can be easily ignored and misdiagnosed in clinic and may bring out severe outcomes. From January 2003 to December 2017, a retrospective analysis of ten patients with epithelioid sarcomas which involved the peripheral nerves was made. The medical data, detailed clinical courses and the follow-up results were stated. In these cases, the tumors invaded the median nerve, ulnar nerve, radial nerve, brachial plexus, sciatic nerve and presented as relative symptoms. Early diagnosis and treatment are the keys to better prognosis. We recommended high-resolution ultrasound as a standard diagnostic tool for nerve compression syndromes not only reveal the morphological structure of the peripheral nerve, but also discover the tumor involving the nerve. Adequate surgical methods including wide resection and lymph node dissection if necessary. The manners of functional reconstruction need to be applied flexibly by the doctors. Postoperative rehabilitation is important for functional recovery. We want to share our experiences in the diagnosis and treatment to overcome this particular condition.

摘要

上皮样肉瘤是罕见的软组织肿瘤,有可能累及周围神经并表现为感觉和运动障碍。其症状与神经压迫性疾病相似。这种情况在临床上极为罕见,仅在文献中有几例病例报告。在临床中它很容易被忽视和误诊,可能会带来严重后果。对2003年1月至2017年12月期间10例累及周围神经的上皮样肉瘤患者进行回顾性分析。陈述了医疗数据、详细的临床病程及随访结果。在这些病例中,肿瘤侵犯正中神经、尺神经、桡神经、臂丛神经、坐骨神经并表现出相应症状。早期诊断和治疗是获得较好预后的关键。我们推荐将高分辨率超声作为神经压迫综合征的标准诊断工具,它不仅能显示周围神经的形态结构,还能发现累及神经的肿瘤。采取充分的手术方法,必要时包括广泛切除和淋巴结清扫。功能重建方式需医生灵活应用。术后康复对功能恢复很重要。我们希望分享我们在诊断和治疗方面的经验以应对这种特殊情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/c994504a7ee8/41598_2024_82357_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/5649f993a619/41598_2024_82357_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/10f6f8b5a170/41598_2024_82357_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/c994504a7ee8/41598_2024_82357_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/5649f993a619/41598_2024_82357_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/10f6f8b5a170/41598_2024_82357_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b16/11680949/c994504a7ee8/41598_2024_82357_Fig3_HTML.jpg

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本文引用的文献

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Subclassification of epithelioid sarcoma with potential therapeutic impact.上皮样肉瘤的亚分类具有潜在的治疗影响。
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Classic epithelioid sarcoma arising from the sciatic nerve: A case report of an uncommon tumor at an unusual site.起源于坐骨神经的经典型上皮样肉瘤:一例罕见部位的罕见肿瘤病例报告。
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超越 SMARCB1 缺失:上皮样肉瘤病理生物学的最新认识。
Cells. 2022 Aug 24;11(17):2626. doi: 10.3390/cells11172626.
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Prognostic nomogram in patients with epithelioid sarcoma: A SEER-based study.上皮样肉瘤患者的预后列线图:基于 SEER 的研究。
Cancer Med. 2023 Feb;12(3):3079-3088. doi: 10.1002/cam4.5230. Epub 2022 Sep 8.
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Classic Type of Epithelioid Sarcoma of the Distal Upper Extremity: Clinical and Oncological Characteristics.经典型肢体远端上皮样肉瘤:临床与肿瘤学特征。
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Epithelioid Sarcoma of the Peripheral Nerve: Clinicopathologic Series of Three Cases and Literature Review.周围神经上皮样肉瘤:三例临床病理系列及文献复习。
Am J Clin Pathol. 2021 Apr 26;155(5):729-737. doi: 10.1093/ajcp/aqaa180.
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Cytopathology of classic type epithelioid sarcoma: a series of 20 cases and review of the literature.经典型上皮样肉瘤的细胞病理学:20 例系列病例及文献复习。
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Classic-type epithelioid sarcoma arising from the sciatic nerve: A case report.起源于坐骨神经的经典型上皮样肉瘤:一例报告
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